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Published on: March 5, 2016

[Early-onset of primary intestinal lymphangiectasia. A case report and diet treatment]

F Greco1, G Piccolo, A Sorge

  • 1Dipartimento di Pediatria, Università degli Studi di Catania, Catania, Italy. filippogreco@tiscali.it

Minerva Pediatrica
|December 17, 2003
PubMed

Insights

Primary intestinal lymphangiectasia, a rare disorder causing protein loss, was effectively treated in an infant using a medium-chain triglyceride (MCT) enriched formula. Early diagnosis and MCT-based treatment led to significant clinical and biochemical improvement.

Area of Science:

  • Gastroenterology
  • Pediatrics
  • Rare Diseases

Background:

  • Primary intestinal lymphangiectasia is a rare condition causing hypoproteinemia.
  • It results from intestinal lymphatic vessel obstruction and lymph fluid loss.
  • Protein-losing enteropathy is a key manifestation.

Observation:

  • A case report details a 3-month-old infant diagnosed with protein-losing enteropathy secondary to primary intestinal lymphangiectasia.
  • Diagnosis was confirmed via duodenal histology.

Findings:

  • The patient initially received an adapted formula.
  • Switching to a formula enriched with medium-chain triglycerides (MCT) resulted in significant clinical and biochemical improvement.

Implications:

  • Early diagnosis of primary intestinal lymphangiectasia is crucial for timely intervention.
  • Medium-chain triglyceride (MCT) enriched formula demonstrates efficacy in managing this condition.
  • This case underscores the therapeutic potential of MCTs in pediatric protein-losing enteropathy.

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